Myotonic muscular dystrophy of early development. Histoenzymologic and ultrastructural findings on muscle biopsies

Scelsi, R.; Besana, D.; Poggi, P.; Lanzi, G.

Acta Neurologica 32(2): 173-188

1977


ISSN/ISBN: 0001-6276
PMID: 930658
Document Number: 111531
Of 7 subjects, 4 had dystrophia myotonica and 3 were born of parents affected with Steinert disease. There was a selective hypotrophy and predominance of type I muscle fibers in dystrophic myotonica. The sarcoplasmic reticulum and t-tubules were dilated and there were sarcoplasmatic masses devoid of myofibrils. The presence of mitochondria, glycogen granules and lysosomes was noted.

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